Mouse anti-Human/Mouse/Rat sapiens (Human) FBXO11 Monoclonal Antibody

Function

Substrate recognition component of a SCF (SKP1-CUL1-F-box protein) E3 ubiquitin-protein ligase complex which mediates the ubiquitination and subsequent proteasomal degradation of target proteins, such as DTL/CDT2, BCL6, SNAI1 and PRDM1/BLIMP1 (PubMed:17098746, PubMed:22113614, PubMed:23478441, PubMed:23478445, PubMed:23892434, PubMed:24613396, PubMed:24968003, PubMed:25827072, PubMed:29059170). The SCF(FBXO11) complex mediates ubiquitination and degradation of BCL6, thereby playing a role in the germinal center B-cells terminal differentiation toward memory B-cells and plasma cells (PubMed:22113614). The SCF(FBXO11) complex also mediates ubiquitination and degradation of DTL, an important step for the regulation of TGF-beta signaling, cell migration and the timing of the cell-cycle progression and exit (PubMed:23478441, PubMed:23478445). The SCF(FBXO11) complex also catalyzes ubiquitination and degradation of GSK3B-phosphorylated SNAI1 (PubMed:25827072, PubMed:29059170). Binds to and neddylates phosphorylated p53/TP53, inhibiting its transcriptional activity (PubMed:17098746). Plays a role in the regulatiom of erythropoiesis but not myelopoiesis or megakaryopoiesis (PubMed:33156908). Mechanistically, activates erythroid genes by mediating the degradation of BAHD1, a heterochromatin-associated protein that recruits corepressors to H3K27me3 marks (PubMed:33156908). Participates in macrophage cell death and inflammation in response to bacterial toxins by regulating the expression of complement 5a receptor 1/C5AR1 and IL-1beta (PubMed:33156908). Acts as a critical regulator to determine the level of MHC-II by mediating the recognition of degron at the P/S/T domain of CIITA leading to its ubiquitination and subsequent degradation via the proteasome (PubMed:37279268). Participates in the antiviral repsonse by initiating the activation of TBK1-IRF3-IFN-I axis (PubMed:36897010). Mediates the 'Lys-63'-linked ubiquitination of TRAF3 to strengthen the interaction between TRAF3 and TBK1 (PubMed:36897010)

Subcellular Location

Nucleus; Chromosome

Disease Association

Intellectual developmental disorder with dysmorphic facies and behavioral abnormalities (IDDFBA) : An autosomal dominant developmental disorder with variable manifestations and onset in infancy or first years of life. Clinical features include intellectual disability, speech delay, hyperkinetic disorder, hyperactivity, seizures, pre- and postnatal growth retardation, microcephaly, and facial dysmorphism. [The disease is caused by variants affecting the gene represented in this entry] | [Defects in FBXO11 may be a cause of diffuse large B-cell lymphoma by allowing the accumulation of BCL6, an oncoprotein that has a critical role in lymphomas]

Tissue Specificity

Isoform 5 is expressed in keratinocytes, fibroblasts and melanocytes

Subunit

Component of the SCF(FBXO11) complex consisting of CUL1, RBX1, SKP1 and FBXO11 (PubMed:23478441, PubMed:23478445). Interacts with CIITA (PubMed:37279268)

Pathway

Protein modification; protein ubiquitination

• Abida W.M. et al. FBXO11 promotes the neddylation of p53 and inhibits its transcriptional activity. — J. Biol. Chem. (2007)
• Mao Y.-M. et al. Isolation of full-length cDNA clones from human fetal brain cDNA library. (FEB-)
• Totoki Y. et al. PubMed: CI-58UKVFP8TE70J (APR-)
• Ota T. et al. Complete sequencing and characterization of 21,243 full-length human cDNAs. — Nat. Genet. (2004)
• Hillier L.W. et al. Generation and annotation of the DNA sequences of human chromosomes 2 and 4. — Nature (2005)
• Mural R.J. et al. PubMed: CI-56FTJV1JKRRTT (SEP-)
• The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). — Genome Res. (2004)
• Cenciarelli C. et al. Identification of a family of human F-box proteins. — Curr. Biol. (1999)